{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T10:18:02Z","timestamp":1787912282984,"version":"build-2784847793"},"reference-count":34,"publisher":"Ovid Technologies (Wolters Kluwer Health)","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>Rising end-diastolic pressure in patients with a Fontan circulation has led to the hypothesis that circulatory failure is driven by progressive ventricular stiffening. However, end-diastolic pressure is influenced by loading conditions and extracardiac constraint and may not accurately reflect chamber stiffness. We aimed to characterize ventricular chamber stiffness using the end-diastolic pressure-volume relationship and examine its clinical and prognostic significance in patients with a Fontan circulation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>\n                      In the FORCE registry (Fontan Outcomes Registry Using Clinical Examinations), chamber stiffness was quantified with the exponential coefficient \u03b2 derived from single-beat end-diastolic pressure-volume relationship reconstruction using paired catheterization and cardiovascular magnetic resonance data. The \u03b2 values were standardized as body surface area\u2013 and sex-based\n                      <jats:italic toggle=\"yes\">Z<\/jats:italic>\n                      scores and related to clinical characteristics, ventricular remodeling, and a composite adverse outcome (death, transplantation listing, sustained atrial or ventricular arrhythmias, protein-losing enteropathy, or plastic bronchitis).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>\n                      Among 814 patients (median age, 14.7 years), median \u03b2 was 0.034 mL\n                      <jats:sup>\u22121<\/jats:sup>\n                      (0.025\u20130.046). Lower \u03b2\n                      <jats:italic toggle=\"yes\">Z<\/jats:italic>\n                      score was associated with dominant right ventricle, eccentric remodeling with reduced ejection fraction, increased collateral flow and intracardiac recirculation, greater symptom burden, and higher arrhythmia prevalence, despite similar filling pressures. Over a median follow-up of 2.7 years (0.9\u20136.1 years), lower \u03b2\n                      <jats:italic toggle=\"yes\">Z<\/jats:italic>\n                      score independently predicted adverse outcomes (adjusted hazard ratio, 1.30 per 1-SD decrease [95% CI 1.04\u20131.61]). A \u03b2\n                      <jats:italic toggle=\"yes\">Z<\/jats:italic>\n                      score &lt;\u22120.71, \u03b2&lt;0.025 mL\n                      <jats:sup>\u22121<\/jats:sup>\n                      , or body surface area\u2013indexed end-diastolic volume &gt;103 mL\/m\n                      <jats:sup>2<\/jats:sup>\n                      showed similarly modest discrimination for adverse outcomes overall (area under the curve, 0.59), with greater discrimination in dominant right ventricles.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>Contrary to prevailing assumptions, lower rather than higher chamber stiffness was associated with adverse Fontan outcomes, identifying a high-compliance phenotype characterized by ventricular dilation and eccentric remodeling. These findings suggest that, in the contemporary FORCE cohort, Fontan failure might be more closely associated with this remodeling phenotype than with progressive stiffening.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.126.081043","type":"journal-article","created":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T10:00:11Z","timestamp":1787911211000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Low Ventricular Chamber Stiffness Identifies a High-Risk Eccentric Remodeling Phenotype in the Fontan Circulation: A FORCE Registry Analysis"],"prefix":"10.1161","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5606-376X","authenticated-orcid":true,"given":"Jef","family":"Van den Eynde","sequence":"first","affiliation":[{"name":"Department of Cardiology, UZ Leuven, Department of Cardiovascular Sciences, KU Leuven, Belgium (J.V.d.E., A.V.D.B.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0469-8640","authenticated-orcid":true,"given":"Alexander","family":"Van De Bruaene","sequence":"additional","affiliation":[{"name":"Department of Cardiology, UZ Leuven, Department of Cardiovascular Sciences, KU Leuven, Belgium (J.V.d.E., A.V.D.B.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Slesnick","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Children\u2019s Healthcare of Atlanta, Emory University School of Medicine, GA (T.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7490-3702","authenticated-orcid":true,"given":"Mark A.","family":"Fogel","sequence":"additional","affiliation":[{"name":"Division of Cardiology, The Children\u2019s Hospital of Philadelphia, University of Pennsylvania (M.A.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1229-3774","authenticated-orcid":true,"given":"Adam L.","family":"Dorfman","sequence":"additional","affiliation":[{"name":"Congenital Heart Center, CS Mott Children\u2019s Hospital, Department of Pediatrics, University of Michigan Medical School, Ann Arbor (A.L.D.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4605-5600","authenticated-orcid":true,"given":"Christopher Z.","family":"Lam","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, ON, Canada (C.Z.L.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1003-1903","authenticated-orcid":true,"given":"Yue-Hin","family":"Loke","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Children\u2019s National Hospital, George Washington University School of Medicine, Washington, DC (Y.-H.L.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9194-5330","authenticated-orcid":true,"given":"Jonathan H.","family":"Soslow","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Vanderbilt University Medical Center, Vanderbilt University School of Medicine, Nashville, TN (J.H.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3777-4822","authenticated-orcid":true,"given":"Tarek","family":"Alsaied","sequence":"additional","affiliation":[{"name":"Children\u2019s Medical Center of Dallas, Dallas, TX (T.A)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7149-5421","authenticated-orcid":true,"given":"Rahul H.","family":"Rathod","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Boston 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